Whipple Disease Clinical Presentation

  • Author: Ingram M Roberts, MD, MBA; Chief Editor: Julian Katz, MD   more...
 
Updated: Dec 2, 2011
 

History

  • The classic presentation of Whipple disease is that of a wasting illness characterized by arthralgias, arthritis, fever, and diarrhea.
  • Lymphadenopathy may be present.
  • If Whipple disease affects the small intestine, steatorrhea often is present.
  • Approximately 90% of patients with Whipple disease present with weight loss, and 70% of patients with Whipple disease complain of either diarrhea or arthralgias.
  • Occult GI bleeding can be found in 80% of patients of Whipple disease, but frank hematochezia is uncommon.
  • Cardiac involvement occurs in approximately 30% of cases.
Next

Physical

  • Swelling of the joints may occur, but frankly deforming arthritis is quite rare.[18] Sacroileitis, pancarpal narrowing, and cervical epiphyseal fusion has been described in selected patients.
  • Patients with Whipple disease may have any of the physical findings associated with malabsorption. These findings are nonspecific but include the following:
    • Cachexia
    • Distended abdomen
    • Glossitis
    • Perlèche (angular cheilitis)
    • Chvostek or Trousseau sign (secondary to hypocalcemia)
    • Gingivitis and parafollicular hemorrhages (secondary to vitamin C deficiency)
    • Night blindness (secondary to vitamin A deficiency)
    • Visible peristalsis with borborygmi
    • Hyperpigmentation around the orbital and malar areas of the face (occasionally)
  • When the CNS is involved, patients may demonstrate signs of frontal release (as seen with dementia), meningoencephalitis, or ataxia and clonus (if the cerebellum is affected).[19, 20] One review noted that supranuclear ophthalmoplegia and cerebellar ataxia were two of the most common neurologic findings.[21]
Previous
Next

Causes

  • The disease is believed to be due to a disordered host response to the bacterium T whippelii. Interestingly, patients with HIV infection do not acquire the disease.
  • Of interest are data that suggest that T whippelii DNA may be found in patients who are asymptomatic.[22] The study revealed its presence in saliva in 35% of a sample of 40 healthy patients.[23] This suggests that Whipple disease is a manifestation of an abnormal host response to a microorganism that may occur frequently in humans (perhaps in a similar manner to that observed with Helicobacter pylori).
  • To date, Koch's postulates have not been fulfilled completely (infection of an animal model and isolation of the organism from the animal). However, T whippelii bacteria have been grown successfully in HEL (a human fibroblast line) cells.[2] The production of immunoglobulin G (IgG) and immunoglobulin M (IgM) antibodies has been shown. The organism has been cultured from affected CSF and vitreous humor of patients with Whipple disease.
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

Ingram M Roberts, MD, MBA  Associate Clinical Professor of Medicine, University of Connecticut School of Medicine; Program Director of Internal Medicine Residency, Vice Chairman, Department of Medicine, St Vincent's Medical Center

Ingram M Roberts, MD, MBA is a member of the following medical societies: American College of Gastroenterology, American College of Physician Executives, American College of Physicians, American Gastroenterological Association, American Medical Informatics Association, American Society for Gastrointestinal Endoscopy, and Association of Program Directors in Internal Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Marco G Patti, MD  Professor of Surgery, Director, Center for Esophageal Diseases, University of Chicago Pritzker School of Medicine

Marco G Patti, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Surgeons, American Gastroenterological Association, American Medical Association, American Surgical Association, Association for Academic Surgery, Pan-Pacific Surgical Association, Society for Surgery of the Alimentary Tract, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, and Western Surgical Association

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Noel Williams, MD  Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

References

  1. Relman DA, Schmidt TM, MacDermott RP, et al. Identification of the uncultured bacillus of Whipple's disease. N Engl J Med. Jul 30 1992;327(5):293-301. [Medline].

  2. Raoult D, Birg ML, La Scola B, et al. Cultivation of the bacillus of Whipple's disease. N Engl J Med. Mar 2 2000;342(9):620-5. [Medline].

  3. Dray X, Vahedi K, Delcey V, et al. Mycobacterium avium duodenal infection mimicking Whipple's disease in a patient with AIDS. Endoscopy. Feb 2007;39 Suppl 1:E296-7. [Medline].

  4. Patel SJ, Huard RC, Keller C, et al. Possible Case of CNS Whipple's Disease in an Adolescent With AIDS. J Int Assoc Physicians AIDS Care (Chic Ill). Jun 2008;7(2):69-73. [Medline].

  5. Ramzan NN, Loftus E Jr, Burgart LJ, et al. Diagnosis and monitoring of Whipple disease by polymerase chain reaction. Ann Intern Med. Apr 1 1997;126(7):520-7. [Medline].

  6. Marth T, Schneider T. Whipple disease. Curr Opin Gastroenterol. Mar 2008;24(2):141-8. [Medline].

  7. Schneider T, Moos V, Loddenkemper C, et al. Whipple's disease: new aspects of pathogenesis and treatment. Lancet Infect Dis. Mar 2008;8(3):179-90. [Medline].

  8. O'Duffy JD, Griffing WL, Li CY, et al. Whipple's arthritis: direct detection of Tropheryma whippelii in synovial fluid and tissue. Arthritis Rheum. Apr 1999;42(4):812-7. [Medline].

  9. Celard M, de Gevigney G, Mosnier S, et al. Polymerase chain reaction analysis for diagnosis of Tropheryma whippelii infective endocarditis in two patients with no previous evidence of Whipple's disease. Clin Infect Dis. Nov 1999;29(5):1348-9. [Medline].

  10. Gubler JG, Kuster M, Dutly F, et al. Whipple endocarditis without overt gastrointestinal disease: report of four cases. Ann Intern Med. Jul 20 1999;131(2):112-6. [Medline].

  11. Gerard A, Sarrot-Reynauld F, Liozon E, et al. Neurologic presentation of Whipple disease: report of 12 cases and review of the literature. Medicine (Baltimore). Nov 2002;81(6):443-57. [Medline].

  12. Kelly CA, Egan M, Rawlinson J. Whipple's disease presenting with lung involvement. Thorax. Mar 1996;51(3):343-4. [Medline].

  13. Dobbins WO 3rd. HLA antigens in Whipple's disease. Arthritis Rheum. Jan 1987;30(1):102-5. [Medline].

  14. Fenollar F, Puéchal X, Raoult D. Whipple's disease. N Engl J Med. Jan 4 2007;356(1):55-66. [Medline].

  15. Durand DV, Lecomte C, Cathebras P, et al. Whipple disease. Clinical review of 52 cases. The SNFMI Research Group on Whipple Disease. Société Nationale Française de Médecine Interne. Medicine (Baltimore). May 1997;76(3):170-84. [Medline].

  16. Keinath RD, Merrell DE, Vlietstra R, et al. Antibiotic treatment and relapse in Whipple's disease. Long-term follow-up of 88 patients. Gastroenterology. Jun 1985;88(6):1867-73. [Medline].

  17. Fleming JL, Wiesner RH, Shorter RG. Whipple's disease: clinical, biochemical, and histopathologic features and assessment of treatment in 29 patients. Mayo Clin Proc. Jun 1988;63(6):539-51. [Medline].

  18. Sheib JS. Whipple disease revisited. Radiographic features of a patient with 35 years of undiagnosed arthritis. J Clin Rheumatol. 2004;10:69-73.

  19. Matthews BR, Jones LK, Saad DA, et al. Cerebellar ataxia and central nervous system Whipple disease. Arch Neurol. Apr 2005;62(4):618-20. [Medline].

  20. Fenollar F, Nicoli F, Paquet C, Lepidi H, Cozzone P, Antoine JC, et al. Progressive dementia associated with ataxia or obesity in patients with Tropheryma whipplei encephalitis. BMC Infect Dis. Jun 15 2011;11:171. [Medline]. [Full Text].

  21. Süzer T, Demirkan N, Tahta K, et al. Whipple's disease confined to the central nervous system: case report and review of the literature. Scand J Infect Dis. 1999;31(4):411-4. [Medline].

  22. Ehrbar HU, Bauerfeind P, Dutly F, et al. PCR-positive tests for Tropheryma whippelii in patients without Whipple's disease. Lancet. Jun 26 1999;353(9171):2214. [Medline].

  23. Street S, Donoghue HD, Neild GH. Tropheryma whippelii DNA in saliva of healthy people. Lancet. Oct 2 1999;354(9185):1178-9. [Medline].

  24. Whistance RN, Elfarouki GW, Vohra HA, Livesey SA. A case of Tropheryma whipplei infective endocarditis of the aortic and mitral valves in association with psoriatic arthritis and lumbar discitis. J Heart Valve Dis. May 2011;20(3):353-6. [Medline].

  25. Swartz MN. Whipple's disease--past, present, and future. N Engl J Med. Mar 2 2000;342(9):648-50. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.